SER recommendations on treatment of refractory Behçet's syndrome.

OBJECTIVE: To develop multidisciplinary recommendations based on available evidence and expert consensus for the therapeutic management of patients with refractory Behçet's syndrome (BS) (difficult to treat, severe resistant, severe relapse) to conventional treatment. METHODS: A group of experts identified clinical research questions relevant to the objective of the document. These questions were reformulated in PICO format (patient, intervention, comparison and outcome). Systematic reviews of the evidence were conducted, the quality of the evidence was evaluated following the methodology of the international working group Grading of Recommendations Assessment, Development, and Evaluation (GRADE). After that, the multidisciplinary panel formulated the specific recommendations. RESULTS: 4 PICO questions were selected regarding the efficacy and safety of systemic pharmacological treatments in patients with BS with clinical manifestations refractory to conventional therapy related to mucocutaneous and/or articular, vascular, neurological parenchymal and gastrointestinal phenotypes. A total of 7 recommendations were made, structured by question, based on the identified evidence and expert consensus. CONCLUSIONS: The treatment of most severe clinical manifestations of BS lacks solid scientific evidence and, besides, there are no specific recommendation documents for patients with refractory disease. With the aim of providing a response to this need, here we present the first official Recommendations of the Spanish Society of Rheumatology for the management of these patients. They are devised as a tool for assistance in clinical decision making, therapeutic homogenisation and to reduce variability in the care of these patients.

Recomendaciones SER sobre el tratamiento del síndrome de Behçet refractario / SER recommendations on treatment of refractory Behçet's syndrome

Objetivo: Elaborar recomendaciones multidisciplinares, basadas en la evidencia disponible y el consenso de expertos, para el manejo terapéutico de los pacientes con síndrome de Behçet refractario (difícil de tratar, resistente grave, recidivante grave) al tratamiento convencional. Métodos: Un panel de expertos identificó preguntas clínicas de investigación relevantes para el objetivo del documento. Estas preguntas fueron reformuladas en formato PICO –paciente, intervención, comparación, outcome o desenlace–. A continuación, se realizaron revisiones sistemáticas; la evaluación de la calidad de la evidencia se realizó siguiendo la metodología del grupo internacional de trabajo Grading of Recommendations, Assessment, Development, and Evaluation. Tras esto, el panel multidisciplinar formuló las recomendaciones. Resultados: Se seleccionaron 4 preguntas PICO relativas a la eficacia y seguridad de los tratamientos farmacológicos sistémicos en los pacientes con síndrome de Behçet con manifestaciones clínicas refractarias a terapia convencional, relacionadas con los fenotipos mucocutáneo y/o articular, vascular, neurológico-parenquimatoso y gastrointestinal. Se formularon un total de 7 recomendaciones estructuradas por pregunta, con base en la evidencia encontrada y el consenso de expertos. Conclusiones: El tratamiento de las manifestaciones clínicas más graves del síndrome de Behçet carece de evidencia científica sólida y no existen documentos de recomendaciones específicas para los pacientes con enfermedad refractaria a la terapia convencional. Con el fin de aportar una respuesta a esta necesidad, se presenta el primer documento de recomendaciones de la Sociedad Española de Reumatología específicas para el abordaje terapéutico de estos pacientes, que servirá de ayuda en la toma de decisiones clínica y la reducción de la variabilidad en la atención.(AU)

Objective: To develop multidisciplinary recommendations based on available evidence and expert consensus for the therapeutic management of patients with refractory Behçet's syndrome (difficult to treat, severe resistant, severe relapse) to conventional treatment. Methods: A group of experts identified clinical research questions relevant to the objective of the document. These questions were reformulated in PICO format –patient, intervention, comparison and outcome–. Systematic reviews of the evidence were conducted; the quality of the evidence was evaluated following the methodology of the international working group Grading of Recommendations, Assessment, Development, and Evaluation. After that, the multidisciplinary panel formulated the specific recommendations. Results: Four PICO questions were selected regarding the efficacy and safety of systemic pharmacological treatments in patients with Behçet's syndrome with clinical manifestations refractory to conventional therapy related to mucocutaneous and/or articular, vascular, neurological parenchymal and gastrointestinal phenotypes. A total of 7 recommendations were made, structured by question, based on the identified evidence and expert consensus. Conclusions: The treatment of most severe clinical manifestations of Behçet's syndrome lacks solid scientific evidence and, besides, there are no specific recommendation documents for patients with refractory disease. With the aim of providing a response to this need, here we present the first official recommendations of the Spanish Society of Rheumatology for the management of these patients. They are devised as a tool for assistance in clinical decision making, therapeutic homogenisation and to reduce variability in the care of these patients.(AU)

Encefalitis límbica secundaria a neuro-Behçet: una presentación insólita / Limbic encephalitis secondary to neuro-Behçet disease: an uncommon presentation

Introducción: La encefalitis límbica (EL) puede tener un amplio abanico de etiologías, más frecuentemente la infecciosa (sobre todo viral) o autoinmune. La enfermedad de Behçet (EB) puede presentarse con manifestaciones neurológicas heterogéneas. Sin embargo, la EL no se considera una presentación típica del neuro-Behçet (NB). Caso clínico: Un varón de 40 años consultó por cefalea de novo subaguda, problemas de memoria y apatía. La anamnesis por sistemas reveló una historia no conocida previamente de aftas orales recurrentes durante años, fiebre y afectación general reciente, así como un episodio de panuveítis bilateral cuatro meses antes de la presentación. Su exploración general y neurológica reveló febrícula, una afta oral aislada, amnesia anterógrada y signos de vasculitis retiniana bilateral. La resonancia magnética mostró un patrón de afectación de meningoencefalitis límbica y su líquido cefalorraquídeo presentaba inflamación mononuclear. El paciente cumplía los criterios diagnósticos de la EB. Considerando que la EL es una presentación muy rara del NB, se buscaron exhaustivamente y se excluyeron otras etiologías alternativas, incluyendo las encefalitis infecciosas, autoinmunes y paraneoplásicas. En consecuencia, el paciente se diagnosticó de NB y mostró una buena recuperación con tratamiento inmunosupresor. Discusión: Sólo dos casos de NB con presentación en forma de EL se han publicado previamente. Comunicamos el tercer caso de esta rara manifestación clínica de la EB y lo comparamos con los dos anteriores, con el objetivo de destacar dicha asociación y contribuir a expandir el rico espectro clínico del NB.(AU)

Introduction: Limbic encephalitis (LE) can have a wide range of etiologies, most frequently infectious (especially viral) or autoimmune. Behçet’s disease (BD) can present with heterogeneous neurological manifestations. However, LE is not considered a typical presentation of neuro-Behçet’s disease (NBD). Case report: A 40-years-old male presented with new-onset subacute headaches, memory problems and apathy. A review of systems revealed an unrecorded past history of recurrent oral sores for years, recent malaise and fever, as well as an episode of bilateral panuveitis four months before presentation. His general and neurologic examination revealed slight fever, an isolated oral aphtha, anterograde amnesia and signs of bilateral retinal vasculitis. Brain magnetic resonance imaging displayed a pattern of limbic meningoencephalitis, and his cerebrospinal fluid showed mononuclear inflammation. The patient met BD diagnostic criteria. Considering LE is a very rare presentation of NBD, alternative etiologies were thoroughly assessed and excluded, including infectious, autoimmune and paraneoplastic encephalitis. Therefore, he was diagnosed with NBD, and he recovered well after immunosuppression. Discussion: Only two cases of NBD presenting with LE have been previously reported. We report a third case of this rare presentation and compare it with the previous two. We aim to highlight this association and contribute to enlarge the rich clinical spectrum of NBD.(AU)

Behçet disease in Latin American countries: A systematic literature review of demographic and clinical features, and HLA-B*51 allele frequency.

OBJECTIVE: To describe the demographic and clinical features, as well as the frequency of the HLA-B*51 allele in Behçet disease (BD) patients in Latin American countries. METHODS: A systematic literature review of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines was conducted without performing a meta-analysis. We included observational studies (cross-sectional or cohort) of BD patients fulfilling the International Study Group for BD classification criteria and reported the demographic, clinical, and laboratory features of the disease in adult patients. RESULTS: Twelve studies were included in the SLR. Information from 532 patients across 5 Latin American countries was included for the analysis. Mean age at disease diagnosis was 33 years, 58.3% were female and 41.7% male; most patients were non-Caucasian. The most common clinical manifestations were recurrent oral ulcers and genital ulcers, followed by skin, eye, joint, neurological, gastrointestinal, vascular, and cardiac involvement. The prevalence of BD was described in 2 studies, 1 conducted in Brazil that reported a prevalence of .3/100,000 inhabitants, and another in Colombia with a prevalence of 1.1/100,000 inhabitants. The frequency of HLA-B*51 allele in BD patients was 38%, 30.1%, and 9% in Argentina, Brazil, and Mexico, respectively. CONCLUSIONS: The prevalence of BD in the Latin American countries seems to be low, as well as the frequency of HLA-B*51 allele. However, the strength of association between HLA-B*51 and BD remains high in our population. The key clinical features of BD are like those reported in countries/regions where BD is endemic.

Behçet disease in Latin American countries: A systematic literature review of demographic and clinical features, and HLA-B*51 allele frequency / Enfermedad de Behçet en países de Latinoamérica: Revisión sistemática de la literatura sobre datos demográficos, hallazgos clínicos y frecuencia del alelo HLA-B*51

Objective: To describe the demographic and clinical features, as well as the frequency of the HLA-B*51 allele in Behçet disease (BD) patients in Latin American countries. Methods: A systematic literature review of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines was conducted without performing a meta-analysis. We included observational studies (cross-sectional or cohort) of BD patients fulfilling the International Study Group for BD classification criteria and reported the demographic, clinical, and laboratory features of the disease in adult patients. Results: Twelve studies were included in the SLR. Information from 532 patients across 5 Latin American countries was included for the analysis. Mean age at disease diagnosis was 33 years, 58.3% were female and 41.7% male; most patients were non-Caucasian. The most common clinical manifestations were recurrent oral ulcers and genital ulcers, followed by skin, eye, joint, neurological, gastrointestinal, vascular, and cardiac involvement. The prevalence of BD was described in 2 studies, 1 conducted in Brazil that reported a prevalence of .3/100,000 inhabitants, and another in Colombia with a prevalence of 1.1/100,000 inhabitants. The frequency of HLA-B*51 allele in BD patients was 38%, 30.1%, and 9% in Argentina, Brazil, and Mexico, respectively. Conclusions: The prevalence of BD in the Latin American countries seems to be low, as well as the frequency of HLA-B*51 allele. However, the strength of association between HLA-B*51 and BD remains high in our population. The key clinical features of BD are like those reported in countries/regions where BD is endemic.(AU)

Objetivo: Describir las características demográficas, clínicas y la frecuencia del alelo HLA-B*51 en pacientes con enfermedad de Behçet (EB) en países de América Latina. Métodos: Se llevó a cabo una revisión sistemática de la literatura (RSL) según la guía PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) sin realizar un metaanálisis final. Se incluyeron estudios observacionales (transversales o de cohortes) de pacientes con EB que cumplieron con los criterios de clasificación del Grupo Internacional de Estudio de la EB e informaron las características demográficas, clínicas y de laboratorio en pacientes adultos con EB. Resultados: Doce estudios fueron incluidos para la RSL. La información de 532 pacientes provenientes de 5 países de América Latina se incluyó para el análisis. La edad media al diagnóstico fue de 33 años, el 58,3% fueron mujeres y el 41,7% hombres; la mayoría de los pacientes fueron no caucásicos. Las manifestaciones clínicas más comunes fueron las úlceras orales y genitales recurrentes, seguidas del compromiso cutáneo, ocular, articular, neurológico, gastrointestinal, vascular y cardíaco. La prevalencia de la EB fue descrita en 2 estudios, uno realizado en Brasil que reportó una prevalencia de 0,3/100.000 habitantes, y otro en Colombia con una prevalencia de 1,1/100.000 habitantes. La frecuencia del HLA-B*51 en pacientes con EB fue del 38%, 30,1% y 9% en Argentina, Brasil y México, respectivamente. Conclusiones: La prevalencia de la EB en los países latinoamericanos parece ser baja, así como la frecuencia del alelo HLA-B*51. Sin embargo, la fuerza de asociación entre el HLA-B*51 y la EB sigue siendo alta en nuestra población. Las características clínicas claves de la EB son similares a las reportadas en países/regiones donde es endémica.(AU)

Aneurisma de la arteria radial que revela una enfermedad de Behçet pediátrica / Radial artery aneurysm showing a paediatric Behçet’s disease

La enfermedad de Behçet es una vasculitis multisistémica cuya etiopatogenia es aún desconocida. Las complicaciones arteriales que tienen una relación directa con la enfermedad en las formas pediátricas son muy raras. Reportamos un caso inusual de la enfermedad de Behçet en un niño de 13 años que fue revelado por la ruptura de un falso aneurisma de la arteria radial. Fue tratado con urgencia por la ligadura arterial seguida de una combinación de corticosteroides y colchicina. A través de este trabajo intentamos averiguar las características de las lesiones arteriales en los niños con enfermedad de Behçet en comparación con la forma adulta.(AU)

Behçet’s disease is a multisystem vasculitis whose aetiopathogenesis is still unknown. Arterial complications directly related to the disease in paediatric forms are very rare. We report an unusual case of Behçet’s disease in a 13-year-old boy who was revealed by a ruptured false aneurysm of the radial artery. He was treated urgently by arterial ligation followed by a combination of corticosteroids and colchicine. Through this work we tried to find out the characteristics of the arterial lesions in children with Behçet’s disease compared to the adult form.

Manifestaciones gastrointestinales de la nfermedad de Behçet / Gastrointestinal manifestations of Behçet´s disease

Resumen La Enfermedad de Behçet (EB), es una entidad poco común, idiopática, de naturaleza crónica y recurrente con afectación multisistémica. Entre ellos, el tracto gastrointestinal ha suscitado especial interés, al asociarse a mayor morbimortalidad y dado sus presentaciones heterogéneas puede simular otras enfermedades gastrointestinales y ser un gran reto diagnóstico para el gastroenterólogo. Por lo tanto, presentamos una revisión narrativa donde se pretenderá describir las manifestaciones gastrointestinales de la EB y sus diagnósticos diferenciales.

Abstract Behçet's Disease (BD) is a rare, idiopathic, chronic and relapsing entity; characterized by multisystem involvement. The gastrointestinal tract has aroused special interest, as it is associated with higher morbidity and mortality and given its heterogeneous presentations, being able to simulate other gastrointestinal pathologies, becoming a great diagnostic challenge for gastroenterologists. Therefore, we present a narrative review, where we intend to describe the possible gastrointestinal manifestations of BD and its differentials.

Clinical course of Covid-19 in a cohort of patients with Behçet disease

Objective: The implications of Covid-19 in patients with Behçet's disease (BD) are unknown. Patients with BD usually take long-term therapy with therapeutic agents that have been tested in Covid-19 patients. We aimed to assess the prevalence of Covid-19 in a cohort of patients with BD and investigate whether those patients with a long-term treatment with colchicine, tumor necrosis factor inhibitors (TNFi) or glucocorticoids are at reduced or increased prevalence of Covid-19 related clinical outcomes.MethodsA retrospective study was conducted among 244 patients with BD (86.1% females; mean age 43.95±11.11 years). Each participant completed an online questionnaire regarding demographics, medical conditions, dispensed colchicine, TNFi or oral glucocorticoids, Covid-19 infection, clinical symptoms and recovery.ResultsThe prevalence of Covid-19 infection was 14.75%. Regarding dose of colchicine, the presence of ageusia was lower in patients taking 0.5mg/day of colchicine compared to those taking 1.5mg/day (p=0.021). The prevalence of dyspnea was significantly higher in patients taking TNFi compared with those without therapy (p=0.032). With regards to oral glucocorticoids, no significant differences were found.ConclusionsThe prevalence of Covid-19 among patients with BD seems to be higher than that among the general population in Spain. Continuous TNFi therapy might increase the prevalence of worse clinical outcomes such as dyspnea; oral glucocorticoids and colchicine apparently provided no protection against the Covid-19 related clinical outcomes of patients with BD. (AU)

Objetivo: Se desconocen las implicaciones de la Covid-19 en pacientes con enfermedad de Behçet (EB). Los pacientes con EB generalmente tienen tratamiento de larga duración con agentes terapéuticos que se han probado en pacientes con Covid-19. Nuestro objetivo fue evaluar la prevalencia de la Covid-19 en una cohorte de pacientes con EB e investigar si los pacientes con un tratamiento de larga duración con colchicina, inhibidores del factor de necrosis tumoral (TNFi) o glucocorticoides tienen una prevalencia reducida o aumentada en los resultados clínicos de la Covid-19.MétodosSe realizó un estudio retrospectivo en 244 pacientes con EB (86,1% mujeres; edad media, 43,95±11,11 años). Cada participante completó un cuestionario en línea sobre datos demográficos, afecciones médicas, tratamiento con colchicina, TNFi o glucocorticoides orales, infección por Covid-19, síntomas clínicos y recuperación.ResultadosLa prevalencia de la infección por Covid-19 fue del 14,75%. En cuanto a la dosis de colchicina, la presencia de ageusia fue menor en los pacientes que tomaban 0,5mg/día de colchicina en comparación con los que tomaban 1,5mg/día (p=0,021). La prevalencia de disnea fue significativamente mayor en los pacientes que tomaban TNFi en comparación con aquellos sin terapia (p=0,032). Con respecto a los glucocorticoides orales, no se encontraron diferencias significativas.ConclusionesLa prevalencia de Covid-19 en pacientes con EB parece ser superior a la de la población general en España. La terapia continua con TNFi podría aumentar la prevalencia de peores resultados clínicos como la disnea; los glucocorticoides orales y la colchicina aparentemente no proporcionan protección contra los resultados clínicos relacionados con la Covid-19 en pacientes con EB. (AU)

Un aneurisma de aorta abdominal roto revela una enfermedad de Behçet / Ruptured abdominal aortic aneurysm reveals Behçet’s disease

Introducción: la enfermedad de Behçet es una vasculitis sistémica de etiología desconocida que afecta generalmente a adultos jóvenes y que puede producir manifestaciones sistémicas (oftálmicas, neurológicas, cardíacas, pulmonares, vasculares y renales) que con frecuencia son la causa final de muerte de estos pacientes. Caso clínico: presentamos el caso clínico de un paciente varón de 56 años con criterios clínicos para el diagnóstico de la enfermedad de Behçet (lesión oral y ulceración genital recurrente) que presentó la rotura de un aneurisma de aorta abdominal. El tratamiento quirúrgico fue mediante resección del aneurisma y reparación de la aorta asociado a terapia inmunosupresora. El posoperatorio cursó con buena evolución. Discusión: la enfermedad de Behçet se asocia con frecuencia a manifestaciones vasculares a diferentes niveles, por lo que el tratamiento debe individualizarse.(AU)

Background: Behçet´s disease is a systemic vasculitis of unknown etiology, which generally affects young adults and can produce systemic manifestations (ophthalmic, neurological, cardiac, pulmonary, vascular and renal), which are often the final cause of death in these patients.Case report: we present the clinical case of a 56-year-old male patient with clinical criteria for the diagnosis of Behçet’s disease (oral lesion and recurrent genital ulceration) who presented a ruptured abdominal aortic aneurysm. Surgical treatment was by resection of the aneurysm and repair of the aorta associated with immunosuppressive therapy. The postoperative period evolved satisfactorily. Disussion: Behçet´s disease is frequently associated with vascular manifestations at different levels, so treatment must be individualized.(AU)

Predictive Factors of Severe Behçet's disease: A Longitudinal, Prospective Cohort Followed Between 1981-2020.

INTRODUCTION: Behçet's disease (BD) is a systemic vasculitis of unknown cause. The spectrum of the disease ranges from mucocutaneous manifestations to other organ diseases with relevant morbidity. Associations between disease severity and male sex, earlier age at onset, and the presence of erythema nodosum have been described. OBJECTIVES: To evaluate clinical factors associated with manifestations of severe disease in a single-center cohort. METHODS: A longitudinal, prospective, unicentric cohort study with patients followed in a specialized outpatient clinic between 1981 and 2020. Severe BD was defined as a Krause total clinical severity score >4 points. RESULTS: We included 243 patients, of whom 31% were male, with an average follow-up time of 14.6 years. Regarding organ manifestations, all patients had mucous manifestations (N=243, 100%), 133 (55%) skin, 104 (43%) joint, 71 (29%) ocular, 48 (20%) vascular, 47 (19%) neurological, 22 (9%) gastrointestinal and 1 (0.4%) cardiac involvement by BD. One hundred fifty-six (64%) patients were classified as having severe BD. Severe BD was more frequent in men (OR=2.004, p=0.024), increasing with age (OR=1.021 per year, p=0.037), in the presence of skin manifestations (OR=4.711, p<0.001), specifically erythema nodosum (OR=8.381, p<0.001), and pseudofolliculitis (OR=2.910, p<0.001). In the multivariate model, variables independently associated with severe BD were male gender (Adjusted OR=1.961, p=0.047), erythema nodosum (Adjusted OR=8.561, p<0.001) and pseudofolliculitis (Adjusted OR=2.372, p=0.007). DISCUSSION: Male gender, erythema nodosum, and pseudofolliculitis were independently associated with severe BD forms and therefore should serve as warning signs to the clinician.

Manejo anestésico de un paciente con enfermedad de Behçet / Anesthetic management of a patient with Behçet´s Disease Sobre los autores:

RESUMEN Introducción: La enfermedad de Behçet es una enfermedad inflamatoria crónica, recurrente, multisistémica, de etiología desconocida, caracterizada por úlceras orales y genitales recurrentes, inflamación ocular, lesiones cutáneas, artritis, afecciones neurológicas, pulmonares, gastrointestinales y vasculitis sistémica. Objetivo: Describir el manejo anestésico en un paciente portador de enfermedad de Behçet. Presentación del caso: Se reporta el caso de un paciente de 52 años de edad con antecedentes patológicos personales de enfermedad de Behçet que recibe anestesia general para exéresis de adenopatía cervical izquierda metastásica de un carcinoma primario oculto. Conclusiones: El mantenimiento del tratamiento con esteroides, el uso de nadroparina cálcica junto a otras medidas preventivas de la trombosis venosa profunda, el manejo cuidadoso de la vía aérea, la protección ocular y la articular, así como de los puntos de presión y la prevención de la patergia son elementos fundamentales en el manejo de estos pacientes.

ABSTRACT Introduction: Behçet's disease is a chronic, recurrent, multisystemic inflammatory disease of unknown etiology characterized by recurrent oral and genital ulcers, ocular inflammation, skin lesions, arthritis, neurological, pulmonary and gastrointestinal conditions, as well as systemic vasculitis. Objective: To describe the anesthetic management of a patient with Behçet's disease. Case presentation: The case is reported of a 52-year-old male patient with an individual history of Behçet's disease, who receives general anesthesia for removal of left cervical metastatic adenopathy from a hidden primary carcinoma. Conclusions: Keeping the steroid therapy, using calcium nadroparin, together with other measures for preventing deep vein thrombosis; careful airway management, eye and joint protection, as well as attention to pressure points and pathergy prevention are fundamental elements for the management of these patients.

Predictive Factors of Severe Behçet's disease: A Longitudinal, Prospective Cohort Followed Between 1981–2020 / Factores predictivos de la enfermedad de Behçet grave: una cohorte prospectiva longitudinal seguida entre 1981-2020

Introduction: Behçet's disease (BD) is a systemic vasculitis of unknown cause. The spectrum of the disease ranges from mucocutaneous manifestations to other organ diseases with relevant morbidity. Associations between disease severity and male sex, earlier age at onset, and the presence of erythema nodosum have been described. Objectives: To evaluate clinical factors associated with manifestations of severe disease in a single-center cohort. Methods: A longitudinal, prospective, unicentric cohort study with patients followed in a specialized outpatient clinic between 1981 and 2020. Severe BD was defined as a Krause total clinical severity score >4 points. Results: We included 243 patients, of whom 31% were male, with an average follow-up time of 14.6 years. Regarding organ manifestations, all patients had mucous manifestations (N=243, 100%), 133 (55%) skin, 104 (43%) joint, 71 (29%) ocular, 48 (20%) vascular, 47 (19%) neurological, 22 (9%) gastrointestinal and 1 (0.4%) cardiac involvement by BD. One hundred fifty-six (64%) patients were classified as having severe BD. Severe BD was more frequent in men (OR=2.004, p=0.024), increasing with age (OR=1.021 per year, p=0.037), in the presence of skin manifestations (OR=4.711, p<0.001), specifically erythema nodosum (OR=8.381, p<0.001), and pseudofolliculitis (OR=2.910, p<0.001). In the multivariate model, variables independently associated with severe BD were male gender (Adjusted OR=1.961, p=0.047), erythema nodosum (Adjusted OR=8.561, p<0.001) and pseudofolliculitis (Adjusted OR=2.372, p=0.007).Discussion: Male gender, erythema nodosum, and pseudofolliculitis were independently associated with severe BD forms and therefore should serve as warning signs to the clinician.(AU)

Introducción: La enfermedad de Behçet (EB) es una vasculitis sistémica de causa desconocida. El espectro de la enfermedad abarca desde manifestaciones mucocutáneas hasta otras enfermedades de órganos con morbilidad relevante. Se han descrito asociaciones entre la gravedad de la enfermedad y el sexo masculino, la edad de inicio más temprana y la presencia de eritema nudoso. Objetivos: Evaluar los factores clínicos asociados con las manifestaciones de enfermedad grave en una cohorte de un solo centro. Métodos: Estudio de cohorte longitudinal, prospectivo y unicéntrico con pacientes seguidos en una clínica ambulatoria especializada entre 1981 y 2020. La EB grave se definió como una puntuación Krause total clinical severity score≥4 puntos. Resultados: Se incluyeron 243 pacientes, de los cuales el 31% eran varones, con un tiempo de seguimiento medio de 14,6 años. En cuanto a las manifestaciones orgánicas, todos los pacientes presentaron manifestaciones mucosas (n=243, 100%), 133 (55%) piel, 104 (43%) articular, 71 (29%) ocular, 48 (20%) afectación vascular, 47 (19%) neurológica, 22 (9%) gastrointestinal y 1 (0,4%) cardiaca por EB; 156 (64%) pacientes fueron clasificados como con EB grave. La EB severa fue más frecuente en hombres (OR=2,004, p=0,024), aumentando con la edad (OR=1,021 por año, p=0,037), en presencia de manifestaciones cutáneas (OR=4,711, p<0,001), específicamente eritema nodosum (OR=8,381, p<0,001) y pseudofoliculitis (OR=2,910, p<0,001). En el modelo multivariado, las variables asociadas de forma independiente con el EB grave fueron el sexo masculino (OR ajustado=1,961, p=0,047), eritema nudoso (OR ajustado=8,561, p<0,001) y pseudofoliculitis (OR ajustado=2,372, p=0,007). Discusión: El sexo masculino, el eritema nudoso y la pseudofoliculitis se asociaron de forma independiente con formas graves de DB y, por lo tanto, deberían servir como signos de advertencia para el médico.(AU)

Are the specific and nonspecific ANA staining patterns of Behçet's Disease patients important?

BACKGROUND: The autoinflammatory character of Behçet's Disease has led researchers to investigate the role of autoantibodies. However, no significant positive result has been reported for autoantibody tests for the disease. AIMS: To investigate the specific and nonspecific staining patterns of Behçet's Disease (BD) patients. METHODS: 140 patients (87 females, 53 males) with an average of 41.9±3 years who were being followed up for Behçet's Disease, and a control group consisting of a total of 736 (464 females, 272 males) healthy volunteers made up of blood donors without any disease whose average age was 50.2±4 years were included in the study. Peripheral venous blood was collected from the patients and the sera were separated. Patient sera were studied by indirect immunofluorescence antibody test (IFA) at a dilution of 1/40 and 1/100. RESULTS: A total of 140 (87 females, 53 males) Behçet's Disease patients and 736 (464 females, 272 males) healthy controls were examined. The rate of ANA positivity was 11.6% in the control group and 10.7% in the Behçet's Disease group. In general, no difference was detected between the patients and the healthy controls in terms of autoantibody positivity (p>0.05). However, when examined in terms of patterns, the low detection of DFS70 and the observation of centriole staining type patterns in Behçet's Disease patients was noteworthy (p<0.05). CONCLUSION: Autoantibody tests, which hold an important place in classic autoimmune diseases, are not necessary for Behçet's patients, but they should be examined in terms of nonspecific patterns.

Are the specific and nonspecific ANA staining patterns of Behçet's Disease patients important? / ¿Son importantes los patrones de tinción ANA específicos y no específicos para los pacientes con enfermedad de Behçet?

Background: The autoinflammatory character of Behçet's Disease has led researchers to investigate the role of autoantibodies. However, no significant positive result has been reported for autoantibody tests for the disease. Aims: To investigate the specific and nonspecific staining patterns of Behçet's Disease (BD) patients. Methods: 140 patients (87 females, 53 males) with an average of 41.9±3 years who were being followed up for Behçet's Disease, and a control group consisting of a total of 736 (464 females, 272 males) healthy volunteers made up of blood donors without any disease whose average age was 50.2±4 years were included in the study. Peripheral venous blood was collected from the patients and the sera were separated. Patient sera were studied by indirect immunofluorescence antibody test (IFA) at a dilution of 1/40 and 1/100. Results: A total of 140 (87 females, 53 males) Behçet's Disease patients and 736 (464 females, 272 males) healthy controls were examined. The rate of ANA positivity was 11.6% in the control group and 10.7% in the Behçet's Disease group. In general, no difference was detected between the patients and the healthy controls in terms of autoantibody positivity (p>0.05). However, when examined in terms of patterns, the low detection of DFS70 and the observation of centriole staining type patterns in Behçet's Disease patients was noteworthy (p<0.05). Conclusion: Autoantibody tests, which hold an important place in classic autoimmune diseases, are not necessary for Behçet's patients, but they should be examined in terms of nonspecific patterns.(AU)

Antecedentes: El carácter autoinflamatorio de la enfermedad de Behçet (EB) ha llevado a los investigadores a estudiar el rol de los autoanticuerpos. Sin embargo, no se ha reportado un resultado positivo significativo para las pruebas de autoanticuerpos. Objetivo: Investigar los patrones de tinción específicos y no específicos de los pacientes con EB. Métodos: Se incluyó en el estudio a 140 pacientes (87 mujeres y 53 varones) con una edad media de 41,9±3 años con seguimiento por EB, y un grupo control que incluyó a un total de 736 voluntarios sanos (464 mujeres y 272 varones) integrados por donantes de sangre sin enfermedad alguna, con una edad media de 50,2±4 años. Se extrajo sangre de vena periférica a todos los pacientes, separándose el suero, que se estudió mediante el test de anticuerpos por inmunofluorescencia directa (IFA) a un factor de dilución de 1/40 y 1/100. Resultados: Se examinó a un total de 140 pacientes (87 mujeres y 53 varones) con EB y 736 controles sanos (464 mujeres y 272 varones). La tasa de positividad de ANA fue del 11,6% en el grupo control y del 10,7% en el grupo de EB. En general no se detectó diferencia entre los pacientes y los controles sanos en términos de positividad de autoanticuerpos (p>0,05). Sin embargo, al realizarse el examen en términos de patrones, fue destacable la baja detección de DFS70 y la observación de los patrones tipo tinción de centriolos en los pacientes con EB (p<0,05). Conclusión: Los test de autoanticuerpos, que ocupan una posición importante en las enfermedades autoinmunes clásicas, no son necesarios para los pacientes con EB, aunque deberían examinarse en términos de patrones no específicos.(AU)

Clinical course of Covid-19 in a cohort of patients with Behçet disease.

OBJECTIVE: The implications of Covid-19 in patients with Behçet's disease (BD) are unknown. Patients with BD usually take long-term therapy with therapeutic agents that have been tested in Covid-19 patients. We aimed to assess the prevalence of Covid-19 in a cohort of patients with BD and investigate whether those patients with a long-term treatment with colchicine, tumor necrosis factor inhibitors (TNFi) or glucocorticoids are at reduced or increased prevalence of Covid-19 related clinical outcomes. METHODS: A retrospective study was conducted among 244 patients with BD (86.1% females; mean age 43.95±11.11 years). Each participant completed an online questionnaire regarding demographics, medical conditions, dispensed colchicine, TNFi or oral glucocorticoids, Covid-19 infection, clinical symptoms and recovery. RESULTS: The prevalence of Covid-19 infection was 14.75%. Regarding dose of colchicine, the presence of ageusia was lower in patients taking 0.5mg/day of colchicine compared to those taking 1.5mg/day (p=0.021). The prevalence of dyspnea was significantly higher in patients taking TNFi compared with those without therapy (p=0.032). With regards to oral glucocorticoids, no significant differences were found. CONCLUSIONS: The prevalence of Covid-19 among patients with BD seems to be higher than that among the general population in Spain. Continuous TNFi therapy might increase the prevalence of worse clinical outcomes such as dyspnea; oral glucocorticoids and colchicine apparently provided no protection against the Covid-19 related clinical outcomes of patients with BD.

Behçet's disease phenotypes and clinical outcomes: A cohort study in egyptian patients.

INTRODUCTION: Behçet's disease (BD) is a systemic inflammatory disease with various presentations. The data on the course of BD in Egyptian patients are limited. OBJECTIVES: The objective of the study was to describe the evolution and association of the different phenotypes of BD. MATERIAL AND METHODS: This chronological cohort study included adult Egyptian patients suffering from BD. Demographic data and the chronological order of the disease's manifestations were collected. RESULTS: The study included 233 patients. Their mean age at the onset of the disease was 26.3±6.9 years. The mean duration from onset of the disease to meeting the criteria was 11.2±30.3 months. The mean duration of the disease was 96.8±72.2 months. On onset of the disease, the most common phenotypes were mucocutaneous (84.5%), musculoskeletal (15.9%), ocular (14.6%) and peripheral venous disease (PVD) (7.3%); on the other hand, pulmonary, peripheral arterial and great vessel phenotypes evolved several years after onset of the disease. The mean time from meeting the criteria to the evolution of a new phenotype was 53.8±58.7 months. Associations between the different phenotypes were observed: PVD and superficial thrombophlebitis, peripheral arterial disease and PVD; another association was also observed between aortic involvement and cerebrovascular disease. CONCLUSION: BD could continue to evolve several years after onset of the disease, making the previous belief about BD yield questionable. BD tends to respect the anatomy of the affected system. Some phenotypes tend to coexist, suggesting a shared aethiopathogeny and that the disease is of a systemic nature.

Behçet's disease phenotypes and clinical outcomes: A cohort study in egyptian patients / Fenotipos y resultados clínicos en la enfermedad de Behçet: un estudio de cohorte en pacientes egipcios

Introduction: Behçet's disease (BD) is a systemic inflammatory disease with various presentations. The data on the course of BD in Egyptian patients are limited. Objectives: The objective of the study was to describe the evolution and association of the different phenotypes of BD. Material and methods: This chronological cohort study included adult Egyptian patients suffering from BD. Demographic data and the chronological order of the disease's manifestations were collected. Results: The study included 233 patients. Their mean age at the onset of the disease was 26.3±6.9 years. The mean duration from onset of the disease to meeting the criteria was 11.2±30.3 months. The mean duration of the disease was 96.8±72.2 months. On onset of the disease, the most common phenotypes were mucocutaneous (84.5%), musculoskeletal (15.9%), ocular (14.6%) and peripheral venous disease (PVD) (7.3%); on the other hand, pulmonary, peripheral arterial and great vessel phenotypes evolved several years after onset of the disease. The mean time from meeting the criteria to the evolution of a new phenotype was 53.8±58.7 months. Associations between the different phenotypes were observed: PVD and superficial thrombophlebitis, peripheral arterial disease and PVD; another association was also observed between aortic involvement and cerebrovascular disease. Conclusion: BD could continue to evolve several years after onset of the disease, making the previous belief about BD yield questionable. BD tends to respect the anatomy of the affected system. Some phenotypes tend to coexist, suggesting a shared aethiopathogeny and that the disease is of a systemic nature.(AU)

Introducción: La enfermedad de Behçet (BD) es una enfermedad inflamatoria sistémica con diversas presentaciones. Los datos sobre el curso de la BD en pacientes egipcios son limitados. Objetivos: El objetivo del estudio fue describir la evolución y la asociación de los diferentes fenotipos de BD. Material y métodos: Este estudio de cohorte cronológico incluyó pacientes egipcios adultos que sufren de BD. Se recopilaron datos demográficos y el orden cronológico de las manifestaciones de la enfermedad. Resultados: El estudio incluyó a 233 pacientes. Su edad media al inicio de la enfermedad fue de 26,3±6,9 años. La duración media desde el inicio de la enfermedad hasta el cumplimiento de los criterios fue de 11,2±30,3 meses. La duración media de la enfermedad fue de 96,8±72,2 meses. Al inicio de la enfermedad, los fenotipos más comunes fueron los fenotipos mucocutáneos (84,5%), musculoesqueléticos (15,9%), oculares (14,6%) y la enfermedad venosa periférica (7,3%); por otro lado, la enfermedad pulmonar, arterial periférica y fenotipos de grandes vasos evolucionaron varios años después del inicio de la enfermedad. La duración media desde el cumplimiento de los criterios hasta la evolución de un nuevo fenotipo fue de 53,8±58,7 meses. Se observaron asociaciones entre los diferentes fenotipos: enfermedad venosa periférica y tromboflebitis superficial, enfermedad arterial periférica y enfermedad venosa periférica; también se observó otra asociación entre la afectación aórtica y la enfermedad cerebrovascular. Conclusión: La BD podría continuar evolucionando varios años después del inicio de la enfermedad, haciendo cuestionable la previa creencia del rendido BD. La BD tiende a respetar la anatomía del sistema afectado. Algunos fenotipos tienden a coexistir, lo que sugiere una etiopatogenia compartida y una naturaleza sistemática de la enfermedad.(AU)

Predictive Factors of Severe Behçet's disease: A Longitudinal, Prospective Cohort Followed Between 1981-2020.

INTRODUCTION: Behçet's disease (BD) is a systemic vasculitis of unknown cause. The spectrum of the disease ranges from mucocutaneous manifestations to other organ diseases with relevant morbidity. Associations between disease severity and male sex, earlier age at onset, and the presence of erythema nodosum have been described. OBJECTIVES: To evaluate clinical factors associated with manifestations of severe disease in a single-center cohort. METHODS: A longitudinal, prospective, unicentric cohort study with patients followed in a specialized outpatient clinic between 1981 and 2020. Severe BD was defined as a Krause total clinical severity score >4 points. RESULTS: We included 243 patients, of whom 31% were male, with an average follow-up time of 14.6 years. Regarding organ manifestations, all patients had mucous manifestations (N=243, 100%), 133 (55%) skin, 104 (43%) joint, 71 (29%) ocular, 48 (20%) vascular, 47 (19%) neurological, 22 (9%) gastrointestinal and 1 (0.4%) cardiac involvement by BD. One hundred fifty-six (64%) patients were classified as having severe BD. Severe BD was more frequent in men (OR=2.004, p=0.024), increasing with age (OR=1.021 per year, p=0.037), in the presence of skin manifestations (OR=4.711, p<0.001), specifically erythema nodosum (OR=8.381, p<0.001), and pseudofolliculitis (OR=2.910, p<0.001). In the multivariate model, variables independently associated with severe BD were male gender (Adjusted OR=1.961, p=0.047), erythema nodosum (Adjusted OR=8.561, p<0.001) and pseudofolliculitis (Adjusted OR=2.372, p=0.007). DISCUSSION: Male gender, erythema nodosum, and pseudofolliculitis were independently associated with severe BD forms and therefore should serve as warning signs to the clinician.

Enfermedad de Behçet frente a la enfermedad inflamatoria intestinal. Diferencias en la presentación clínica / Behçet's disease compared with inflammatory bowel disease. Differences in their clinical presentation

Resumen La enfermedad de Behçet es una enfermedad poco frecuente con un compromiso sistémico cuyas principales manifestaciones abarcan el sistema gastrointestinal, neurológico, vascular y articular, además de la piel y los ojos. Por su compromiso multisistémico, es frecuente confundirlo con otras entidades como la enfermedad inflamatoria intestinal, con la cual comparte gran parte de su presentación clínica, por lo que se describe un caso clínico y se aborda una revision narrativa desde el punto de vista epidemiológico de las manifestaciones clínicas con el fin de tener un mayor conocimiento para identificarla a tiempo.

Abstract Behçet's disease is a rare disease with systemic involvement whose main manifestations include the gastrointestinal, neurological, vascular, and articular systems, as well as the skin and eyes. Due to its multisystemic nature, it is often mistaken for other entities such as inflammatory bowel disease since they share multiple characteristics in their clinical presentation. A clinical case is described, and an epidemiological review of clinical manifestations is discussed to have better knowledge that allow identifying it on time.

Quality of Life in Patients With Behçet Disease and Its Relation With Clinical Symptoms and Disease Activity / Calidad de vida en pacientes con enfermedad de Behçet y su relación con los síntomas clínicos y la actividad de la enfermedad

Background: Behçet's disease(BD) is a systemic vasculitis which may affect the quality of life (QoL).AimsThis study aimed to assess the QoL in Iranian Azeri BD patients as well as evaluating the association between QoL and disease characteristics including disease activity and clinical symptoms. Methods: In this cross-sectional study, a total of 123 BD patients aged 15–65 years fulfilling International Criteria for BD and 123 healthy controls matched with regard to gender were included. Patients aged 15–18 years had the informed consent and consent of their parents for participation. Short Form-36 Quality of Life Scale (SF-36) was used to evaluate QoL. Disease activity was measured by Behcet's Disease Current Activity Form and the Iranian Behcet's Disease Dynamic Activity Measure and Vasculitis Damage Index (VDI) was used for the clinical assessment of damage. Results: The mean±SD age of BD patients and control group was 38.19±10.99 and 33.65±7.29 years, respectively. The Physical Health score as well as most of SF-36 domains including Role Physical, Bodily Pain, General Health, Vitality, and Mental Health were significantly lower in BD patients compared with the control group (P<0.05). There was significant correlation between the Physical and Mental Health scores and most of SF-36 domains with disease activity and clinical symptoms including genital ulcers, ophthalmic and central nervous system (CNS) involvement (P<0.05). Significant correlation was only observed between VDI and Bodily Pain domain (P<0.05). Conclusion: Quality of life is impaired in BD patients, and this impairment is related with disease severity. It seems that presence of genital ulcers, eye and CNS involvement lead to the impairment of QoL in BD patients. These results highlight the importance of nursing interventions in managing clinical symptoms in these patients.(AU)

Antecedentes: La enfermedad de Behçet (BD) es una vasculitis sistémica que puede afectar la calidad de vida (QoL).ObjetivosEste estudio tuvo como objetivo evaluar la QoL en pacientes con BD azerí iraní, así como evaluar la asociación entre la QoL y las características de la enfermedad, incluida la actividad de la enfermedad y los síntomas clínicos. Métodos: En este estudio transversal, un total de 123 pacientes con BD de 15 a 65 años de edad cumplieron con los criterios internacionales para BD y 123 controles sanos que fueron emparejados por género. Los pacientes de 15 a 18 años de edad tenían el consentimiento informado y el consentimiento de sus padres para participar en el estudio. Se usó la escala de calidad de vida corta-36 (SF-36) para evaluar la calidad de vida. La actividad de la enfermedad se midió mediante el formulario de actividad actual de la BD y la medida de la actividad dinámica de la BD iraní y el índice de daño de la vasculitis (VDI) se usaron para la evaluación clínica del daño. Resultados: La media±DE de los pacientes con BD y el grupo control fue de 38,19±10,99 y 33,65±7,29 años, respectivamente. La puntuación de Salud física, así como la mayoría de los dominios SF-36, incluyendo Rol físico, Dolor corporal, Salud general, Vitalidad y Salud mental, fueron significativamente más bajos en los pacientes con BD en comparación con el grupo control (p<0,05). Hubo una correlación significativa entre los puntajes de Salud física y mental y la mayoría de los dominios SF-36 con actividad de la enfermedad y síntomas clínicos, como úlceras genitales, compromiso del sistema oftálmico y del sistema nervioso central (SNC) (p<0,05). Solo se observó una correlación significativa entre el VDI y el dominio del dolor corporal (p<0,05). Conclusión: La calidad de vida se ve afectada en los pacientes con BD, y este deterioro está relacionado con la gravedad de la enfermedad.(AU)